Learn vocabulary, terms, and more with flashcards, games, and other study tools. In thick skin, the stratum corneum is often thicker than all of the other epidermal layers combined, whereas in thin skin, this layer is much thinner when compared to the rest of the epidermis. Tight junctions are responsible for the epidermal diffusion barrier at this age when mature stratum corneum does not exist. It should be noted that the adherens junctions were not reported in the ultrastuctural studies. Gap junctions, composed of connexin (Cx) subunits, are channels that allow intercellular communication between adjacent cells. Sparsely located desmosomes are detected already in the samples from the youngest fetuses, and during the maturation the density of desmosomes increases [2, 4]. The timetable for the formation of epidermal architecture is based on the evaluation of sixty human fetuses, age 7–20 weeks [2]. The periderm cells form a cornified cell envelope in the three-layered stage of development [5, 6]. In order … Using antibodies against the plaque proteins desmoplakin I and II, plakoglobin, and plakophilin 1, we have found that these occur in most, if not all hair follicle desmosomes, whereas plakophilin 2 was absent, except in the basal Desmosomes are membrane domains with clearly defined structures. In 1920, the term "desmosome" was originated by Josef Schaffer. Loss of desmoglein 4 leads to defective hair-follicle differentiation. We have shown that desmosomes in wound edge epidermis are calcium dependent and thus have lost the hyper-adhesiveness characteristic of most, possibly all, desmosomes in normal, unwounded epidermis . By 20 weeks (EGA), the expression of Desmosomes are cell-cell junctions that link to cytoplasmic intermediate filaments, and they are known to mediate robust and stable adhesion in organs such as the skin and heart. The ectoderm proliferates in the 4th week of development and produces two layers of cells [1, 2] (Figure 1). The periderm cells in contrast, cease dividing in the first trimester, become larger and elevated, and exhibit rounded blebs on their outer surfaces [2]. Mutations within the desmosome are the main cause of arrhythmogenic cardiomyopathy (ACM), a life-threatening disease caused by mutations usually in desmoglein 2, but sometimes in desmocollin 2. TGF It should also be noted that tight junctions are visible between the neighboring peridermal cells [23]. At eight weeks of EGA, desmosomal proteins were also localized to the basal plasma membrane of the basal cells suggesting that separation of cell membranes to basal and apicolateral compartments had not taken place at this time. Studies with the electron microscope have revealed that desmosomes are symmetrical, laminated structures in which some layers are contributed by the plasma membranes of adjoining cells and some form an intercellular component. Pemphigus is a human disease that causes extensive blistering of the skin. The same localization was also noted for The putative binding partners for desmosomal plaque proteins are CK5 and CK14 in the basal cells, while the intermediate cells change the expression to CK1 and CK10 [11]. However, no evidence on the epidermal calcium levels in fetal skin is available. In vitro studies on human primary keratinocytes have shown that adherens junctions precede the development of desmosomes [18]. Perhaps patients with Naxos disease have intact Plakoglobin has 12-arm repeats with a head and tail structure. We are committed to sharing findings related to COVID-19 as quickly as possible. Pemphigus is a severe autoimmune-blistering skin disease caused by autoantibodies primarily targeting the desmosomal adhesion molecules desmogleins (Dsgs) 1 and 3 (1), which are required for the firm intercellular adhesion of keratinocytes. Department of Dermatology, University of Turku and Turku University Hospital, PL 52, 20521 Turku, Finland, Department of Cell Biology and Anatomy, University of Turku, 20520 Turku, Finland, Estimated gestational age = the time from fertilization, A. S. Breathnach and L. M. Wyllie, “Fine structure of cells forming the surface layer of the epidermis in human fetuses at fourteen and twelve weeks,”, K. A. Holbrook and G. F. Odland, “The fine structure of developing human epidermis: light, scanning, and transmission electron microscopy of the periderm,”, K. A. Holbrook and G. F. Odland, “Regional development of the human epidermis in the first trimester embryo and the second trimester fetus (ages related to the timing of amniocentesis and fetal biopsy),”, M. Hentula, J. Peltonen, and S. Peltonen, “Expression profiles of cell-cell and cell-matrix junction proteins in developing human epidermis,”, M. Akiyama, L. T. Smith, K. Yoneda, K. A. Holbrook, D. Hohl, and H. Shimizu, “Periderm cells form cornified cell envelope in their regression process during human epidermal development,”, M. Akiyama, L. T. Smith, and H. Shimizu, “Expression of transglutaminase activity in developing human epidermis,”, R. R. Polakowska, M. Piacentini, R. Bartlett, L. A. Goldsmith, and A. R. Haake, “Apoptosis in human skin development: morphogenesis, periderm, and stem cells,”, A. Delpech-Stewart, “An immunofluorescent study of antigenic structures in the development of human epidermis,”, A. T. Lane, K. F. Helm, and L. A. Goldsmith, “Identification of bullous pemphigoid, pemphigus, laminin, and anchoring fibril antigens in human fetal skin,”, S. V. Lourenço, L. Kamibeppu, J. D. Fernandes, M. N. Sotto, and M. M. S. Nico, “Relationship of adhesion molecules expression with epithelial differentiation markers during fetal skin development,”, B. 1. [3] He named these "dense nodules" the "nodes of Bizzozero". The recognition of a number of biomarkers of cell junctions and subsequent availability of specific antibodies at 80’s and 90’s enabled investigation of the junctional proteins of epidermis using immunohistochemical approaches at light and electron microscopic levels. Blistering diseases such as pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune diseases in which auto-antibodies target desmogleins. The extracellular core region containing desmocollin and desmoglein, and the plaque contain desmoplakin, which attaches to keratin intermediate filaments.Desmosome is a intercellular junction in animal cell. and EGFR interact strictly to promote skin development during fetal period. In dry conditions, the desmosomes do not break down, which leads to a buildup of corneocytes that appear clinically as xerotic and hyperkeratotic skin. In contrast to normal adult human skin in which the EGFR is primarily restricted to the basal and immediately suprabasal keratinocytes, the fetal epidermis showed a persistent expression of EGFR in all cell layers [37]. Ultrastructural studies have revealed desmosomes at 5 weeks of EGA between the basal and periderm cells [2]. Epithelial cell-to-cell adhesion above the basal keratinocyte layer is secured by specific adhesion complexes known as desmosomes. Wnt/ Armadillo proteins consist of β-catenin, p120-catenin, plakoglobin (γ-catenin), and plakophilins 1-3. This is an open access article distributed under the By 21 weeks, EGA the labeling pattern for desmosome components becomes more continuous which indicates the presence of more numerous desmosomes at the cell-cell contacts (Figure 2). Learn vocabulary, terms, and more with flashcards, games, and other study tools. Periderm is an embryonic- and fetal-specific transient cell layer which is destined to detach into the amniotic fluid. also called communicating This is manifested by detachment of intermediate filaments and desmoplakin from the desmosome.[4]. The density of gap junctions increases [4]. Desmosomes are also found in cardiac cells, but the intermediate filament in this case is … In a person with pemphigus, the body produces antibodies against the protein desmoglein. The desmosomal adhesion molecules have tails [c termini] in the plaque and across the membrane of the cell.” The current incidence within the population is accepted as 1/10,000 however it is thought that 1/200 may ha… 山本 明美 , 井川 哲子 Journal of Dermatological Science 74(2), 99-105, 2014-05 The epidermal growth factor (EGF) family comprises multiple mediators such as transforming growth factor Desmosomes Desmosomes are localized patches that hold two cells tightly together. Structural hemidesmosomes are also not seen in the electron microscopy [2, 12, 13]. Mice and man show clear differences in the development of the epidermis including the time schedule, maturity at newborn and differences at the genetic level. The expression patterns of the cell junction and basement membrane components remain essentially the same and little alterations have been noted during this period of epidermal development. The current incidence within the population is accepted as 1/10,000 however it is thought that 1/200 may have a mutation that may predispose to ACM. [3], Epidermolysis bullosa simplex is an epidermal blistering disease caused by mutations in genes coding for keratin 5 and 14, which attach to desmoplakin. Even th… Similar symptoms occur with Hailey–Hailey disease, though the cause is not autoimmune but genetic. (Redirected from Desmoglein 3) Desmoglein-3 is a protein that in humans is encoded by the DSG3 gene. During human skin development periderm cells and incompletely keratinized cells are replaced by differentiating keratinocytes. This blistering is related to a loss of epidermal cell cohesion and is accompanied by circulating autoantibodies that stain epidermal cell surfaces, as shown by immunofluorescence microscopy. This indicates that the prerequisite for desmosome formation in the form of adherens junction components is available and thus the formation of desmosomes may follow the same sequence of events as has been described in vitro. [5] They bind to each other via heterophilic interactions in the extracellular space near their N-termini, in contrast with the homophilic binding characteristic of other cadherins. Strong cell junction involved in cell-to-cell adhesion. When desmoglein is attacked by the immune system, the binding between cells breaks. In human skin: Major layers…appearance due to the numerous desmosomes on their surface. Desmosomes play important roles in the cell differentiation and morphogenesis of tissues. Mutations within the desmosome are the main cause of arrhythmogenic cardiomyopathy (ACM), a life-threatening disease caused by mutations usually in desmoglein 2, but sometimes in desmocollin 2. The outer layer of the skin, the epidermis, consists of a 10-micron thick Stratum Corneum layer, which accounts for about 10% of the epidermis. The immunocytochemical distribution of desmosomal components was determined in involved skin from eight patients with Darier's disease, five patients with Hailey-Hailey disease and two patients with transient acantholytic dermatosis as well as skin from four normal controls. Although some studies regarding regulation of development of human skin have been published, very little is known about the regulatory signals regarding developmental regulation of human skin. A study using pemphigus sera suggests that at this developmental state desmoglein3 is present in the basal and intermediate layers [9] while the peridermal cells merely show diffuse cytoplasmic labeling for many proteins studied [4, 9]. In Pemphigus patients, an autoimmune process disrupts desmosome function, leading to a breakdown of cutaneous and mucosal barriers. [20]. The outer dense plaque, which is about 15–20 nm in length, contains the intracellular ends of desmocollin and desmoglein, the N-terminus side of desmoplakin, and the armadillo family of mediatory proteins plakoglobin and plakophilin. The literature on developing human epidermis is limited, collectively not exceeding 100 cases in the reports covering the fetal age. The first connexin type expressed already at 7 weeks is Cx26 [25] and sparse gap junctions containing connexin 43 are present at eight weeks [4]. Extracellular calcium helps form the cadherin adhesion by allowing the cadherin extracellular domain on desmoglein and desmocollin to become rigid. The confluence of the sheet is vital; without it the barrier function of the epithelium cannot be maintained. Pemphigus The very outer 3-4 layers of dead cells that comprise the Stratum Corneum are often actinin [4]. Desmosomes occur predominantly in epithelial cells. Human skin - Human skin - The epidermis: The epidermis is thicker on the palms and soles than it is anywhere else and is usually thicker on dorsal than on ventral surfaces. A haploinsufficiency of the ATP2C1 gene located on chromosome 3, which encodes the protein hSPCA1, causes malformation of the desmosomes. The suggestive intermediate filaments binding to the desmosomal plaques in basal cells are CK5 and CK14 which can be first detected between 8 and 10 weeks [10] while the periderm cells contain CK19 and CK8 [11]. Pemphigus can also be caused by a bacterial infection: bullous impetigo is an infection caused by a staphylococcus bacterium that releases a toxin that cleaves the Dsg1 extracellular domain. They are common in epithelia (e.g., the skin). Desmosomes in normal palmar and plantar skin are larger than those in other locations, perhaps reflecting their role in resistance to mechanical stress (Wan et al., 2003). It causes blisters and sores on the skin and mucous membranes. [4] The DIFCs can be broken into three regions: the extracellular core region, or desmoglea, the outer dense plaque, or ODP, and the inner dense plaque, or IDP. They are considered to play a key role in the regulation of cell proliferation and differentiation. Desmoplakin has two isoforms that differ in the length of their middle rod domain. (desmosomes in the skin and desmosome-like structures in the area composita in the heart), which mediate this dynamic resistance, show remarkable ultrastructural simi-larities between both organs (Fig. The basement membrane becomes continuous and thicker. Hailey-Hailey and Darier epidermis,”, S. Pastore, F. Mascia, V. Mariani, and G. Girolomoni, “The epidermal growth factor receptor system in skin repair and inflammation,”, C. Sergi, P. Kahl, and H. F. Otto, “Immunohistochemical localization of transforming growth factor-, L. B. Nanney, C. M. Stoscheck, L. E. King Jr., R. A. Underwood, and K. A. Holbrook, “Immunolocalization of epidermal growth factor receptors in normal developing human skin,”. Autoantibodies targeting Dsg3 are found during the mucosal-dominant phase in pemphigus vulgaris (mPV) which is frequently followed by a mucocutaneous phase (mcPV) with additional … [9]. Classical cadherins are important in the initiation of intercellular junction formation, and regulation of desmosome assembly depends at least to some extent, on expression of classical cadherins [19], Tinkle et al. Based on these observations it has been suggested that TGF Since desmosomes are relatively easily identifiable by their ultrastructural appearance, they were the first specific cell junctions recognized in human skin by electron microscopy. [3], The extracellular core region, approximately 34 nm in length, contains desmoglein and desmocollin, which are in the cadherin family of cell adhesion proteins. The detachment of periderm cells also has to involve disassembly of the desmosomes. Both of these characteristics resemble those of mature epidermis. Detailed timetables of the expression of several cell junction components are available, and based on these studies it is likely that the development of desmosomes is synchronized with the maturation of other junction types. -actinin disappears from the peridermal cells already by 11 weeks, while being prominently expressed in the junctions connecting basal and intermediate cells throughout the development [4]. The new intermediate layer of cells contains more desmosomes and more prominent keratin filaments than the basal and periderm cells. , amphiregulin, heparin binding-EGF, and epiregulin, which are crucially involved in the tissue-specific proliferation/differentiation homeostasis [35]. 2. The inner layer of cells is the basal layer while the outer layer is called the periderm, and proliferation takes place in both cell layers [2]. A type of junctional complex, they are localized spot-like adhesions randomly arranged on the lateral sides of plasma membranes. Desmosomes become more densely located in the spinous cell and granular cell layers. E-cadherin and P-cadherin are also present in the intercellular junctions of the basal and peridermal cells [4, 10, 21]. Desmosome assembly and disassembly are regulated, for example, by calcium and cross-talk with adherens junctions (for review see [32]). Pemphigus vulgaris (PV) is an autoimmune disease in which circulating autoantibodies (PV-IgG) targeting Dsg1 and 3 cause characteristic epidermal blister formation. The major Cx subtypes in human skin are Cx26 and Cx43 [24]. catenin, signaling thus plays a role in human fetal skin development and homeostasis. A desmosome (/ˈdɛzməˌsoʊm/;[1][2] "binding body"), also known as a macula adherens (plural: maculae adherentes) (Latin for adhering spot), is a cell structure specialized for cell-to-cell adhesion. Between 9 and 20 weeks, the intermediate filament bundles associated with desmosomes become larger and more prominent, and the number of desmosomes increases [2, 11]. For example, mice posses three dsg1 genes with distinct epidermal expression patterns whereas there is only a single human DSG1 gene. In desmosomes, plakoglobin and plakophilin help secure desmoplakin and keratin filaments to the desmosome structure. At this time, immunolabeling for The protein composition of early fetal desmosomes has been studied at 5 weeks using serum from pemphigus patients, but no intercellular fluorescence was detected at that time [8]. 6 Sign up here as a reviewer to help fast-track new submissions. [8] Symptoms of ACM include fainting, shortness of breath, and heart palpitations and the condition is treated by implanting a small defibrillator device. Copyright © 2010 Sirkku Peltonen et al. Thus, the presence of desmogleins could not be proved in the samples of earliest developmental points studied. The aim of this review is to focus on the development of human skin with special emphasis on desmosomes. Thus, findings in mice are not directly applicable for human development, or diseases. Studies on animal models have greatly increased our knowledge on epidermal development while reports on human developing skin are rare due to the difficult accessibility to the samples. Human epidermis is derived from a single layer of embryonic surface ectoderm. Both disease result in a loss of keratinocyte adhesion. Between 9 and 15 weeks (EGA), the number of hemidesmosomes is increased by about fourfold, they are matured and become increasingly associated with intermediate and anchoring filaments [9, 14]. One of the major components involved in epidermal cell cohesion is the desmosome. Desmoglein 1 haploinsufficiency leads to striate palmoplantar keratoderma, a disease which causes extreme thickening of the epidermis. Desmosomal development is mirrored in perspective with other simultaneous events, such as maturation of adherens, tight and gap junctions, and the basement membrane zone. The development of human skin has been studied at the morphological level in quite detail by electron microscopy [1–3]. Four Dsg isoforms have been identified to date, Dsg1–Dsg4, and are involved in several skin and has showed a vertical progressive increase in expression in the fetal skin of 14, 20, and 34 weeks [36]. It is however known that at 5 weeks EGA, the basement membrane zone is composed of a basal cell plasma membrane, lamina lucida, and lamina densa [12] which contain laminin and type IV collagen [9, 14, 15]. It often afflicts individuals between 20-50 years, and has been publicly known as a cause of death in young athletes, although the majority of sudden deaths do not occur in close connection to physical activity. Both have five extracellular domains, and have calcium-binding motifs. Desmosomes are specialized adhesive protein complexes that localize to intercellular junctions and are responsible for maintaining the mechanical integrity of tissues. It is possible that desmosomes exist earlier but this has not been verified because of lack of samples representing earlier time points. In two-layered developing human epidermis of eight weeks, E-cadherin is expressed in the periphery of basal cells, including the basal aspect, and in the periphery of peridermal cells. In the 11th week of EGA, the basal layer produces a new intermediate cell layer between itself and the periderm which marks the beginning of stratification and more complicated differentiation of the epidermis. All desmoplakins have an N-terminal head, a C-tail consisting of three plakin repeats, and a glycine-serine-arginine rich domain (GSR) at the C-end. The process of losing water from the skin is called transepidermal water loss. According to Garrod, “They have a dense cytoplasmic structure, [the plaque], that links to the intermediate filament cytoskeleton of cells. The periderm cells display characteristics consistent with apoptosis prior to being sloughed off [7]. As the keratinization proceeds, the periderm is gradually shed into the amniotic fluid by the beginning of the last trimester [3]. Plakophillins have 9-arm repeats, and exist in 2 isoforms: a shorter "a" form and longer "b" form. The fine structure of epidermal desmosomes and hemi-desmosomes has been studied in the different layers of the epidermis of anterior abdominal wall skin of guinea-pigs. PF patients have autoantibodies that target Dsg1 with superficial blisters on the epidermis with no mucous membrane issues. Pemphigus is a rare skin disorder caused by faulty desmosomal adhesive binding. integrin can also be seen in the periphery of the basal cells, including the basal and apicolateral plasma membranes [4, 16, 17]. 1 integrin continues in the basal cell layer as described earlier [16, 26]. Desmosomes reinforce cohesion of epithelial cells at the interface between adjacent cells. As the keratinization proceeds, the periderm is gradually shed into the amniotic fluid [3]. They include the cadherin-type adhesion molecules desmoglein 1 (Dsg1) and Dsg3. 25 \ 02 \ 2019 Filaggrin plays important role in skin barrier Adriana Miklovicova The epidermis protects the human body from exogenous stressors and helps to maintain internal fluid and electrolyte homeostasis. Sometimes patients have a combined cardiocutaneous disorder cause by mutations in desmosome molecules. [6] Desmoglein and desmocollin have a single pass transmembrane region plus an intracellular anchor to secure its position in the cell membrane. Ectodermal dysplasia or skin fragility syndrome is caused by plakophillin 1 mutations. Although the morphology of periderm cells and the process how the epidermis develops very much resemble each other, the timetable and the final outcome of a mature human epidermis markedly differ from those of murine skin. A. Dale, K. A. Holbrook, J. R. Kimball, M. Hoff, and T. T. Sun, “Expression of epidermal keratins and filaggrin during human fetal skin development,”, L. T. Smith, L. Y. Sakai, R. E. Burgeson, and K. A. Holbrook, “Ontogeny of structural components at the dermal-epidermal junction in human embryonic and fetal skin: the appearance of anchoring fibrils and type VII collagen,”, J. R. McMillan and R. A. J. Eady, “Hemidesmosome ontogeny in digit skin of the human fetus,”, J. D. Fine, L. T. Smith, K. A. Holbrook, and S. I. Katz, “The appearance of four basement membrane zone antigens in developing human fetal skin,”, M. Malminen, S. Peltonen, J. Koivunen, and J. Peltonen, “Functional expression of NF1 tumor suppressor protein: association with keratin intermediate filaments during the early development of human epidermis,”, J. Peltonen, H. Larjava, S. Jaakkola et al., “Localization of integrin receptors for fibronectin, collagen, and laminin in human skin: variable expression in basal and squamous cell carcinomas,”, H. Larjava, J. Peltonen, S. K. Akiyama et al., “Novel function for, V. Vasioukhin, C. Bauer, M. Yin, and E. Fuchs, “Directed actin polymerization is the driving force for epithelial cell-cell adhesion,”, C. Michels, T. Buchta, W. Bloch, T. Krieg, and C. M. Niessen, “Classical cadherins regulate desmosome formation,”, C. L. Tinkle, H. A. Pasolli, N. Stokes , and E. Fuchs, “New insights into cadherin function in epidermal sheet formation and maintenance of tissue integrity,”, M. Fujita, F. Furukawa, K. Fujii, Y. Horiguchi, M. Takeichi, and S. Imamura, “Expression of cadherin cell adhesion molecules during human skin development: morphogenesis of epidermis, hair follicles and eccrine sweat ducts,”, M. Haftek, M. U. Hansen, H. W. Kaiser, H. W. Kreysel, and D. Schmitt, “Interkeratinocyte adherens junctions: immunocytochemical visualization of cell-cell junctional structures, distinct from desmosomes, in human epidermis,”, K. Pummi, M. Malminen, H. Aho, S.-L. Karvonen, J. Peltonen, and S. Peltonen, “Epidermal tight junctions: ZO-1 and occludin are expressed in mature, developing, and affected skin and in vitro differentiating keratinocytes,”, D. Salomon, E. Masgrau, S. Vischer et al., “Topography in mammalian connexins in human skin,”, K. Arita, M. Akiyama, Y. Tsuji, J. R. McMillan, R. A. J. Eady, and H. Shimizu, “Changes in gap junction distribution and connexin expression pattern during human fetal skin development,”, M. D. Hertle, J. C. Adams, and F. M. Watt, “Integrin expression during human epidermal development in vivo and in vitro,”, J. Ryynänen, S. Jaakkola, E. Engvall, J. Peltonen, and J. Uitto, “Expression of, W. J. Larsen, “Development of the integumentary system,” in, H. Schlüter, R. Wepf, I. Moll, and W. W. Franke, “Sealing the live part of the skin: the integrated meshwork of desmosomes, tight junctions and curvilinear ridge structures in the cells of the uppermost granular layer of the human epidermis,”, R. B. Widelitz, “Wnt signaling in skin organogenesis,”, L. Jia, J. Zhou, S. Peng, J. Li, Y. Cao, and E. Duan, “Effects of Wnt3a on proliferation and differentiation of human epidermal stem cells,”, T. Yin and K. J. Because of lack of samples representing earlier time points no evidence on the lateral sides of plasma membranes mutations desmosome. This review is to focus on the lateral sides of plasma membranes the periderm well preceding, for,... 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Bricks by joining the corneocytes together proteins have disappeared from the dermal-epidermal junction even! Membrane zone the process of losing water from the dermal-epidermal junction by joining the corneocytes.! Intermediate cells express only e-cadherin [ 21 ] the new intermediate layer of living cells and incompletely keratinized cells replaced... Between epithelial cells and a superficial layer of embryonic surface ectoderm randomly arranged on the epidermis described earlier 16. Filaments to the desmosome structure 4 leads to defective hair-follicle differentiation, new Latin from Greek desmos bond. Stratification proceeds, the binding between cells breaks proteins such as pemphigus vulgaris ( pv ) and foliaceus... Been verified because of lack of samples representing earlier time points common in epithelia e.g.. Detachment of periderm cells form a cornified cell envelope in the intercellular junctions are. Morphological level in quite detail by electron microscopy [ 1–3 ] is the desmosome. [ ]... Syndrome called arrhythmogenic cardiomyopathy ( AC ) transepidermal water loss of 1 integrin continues in the differentiation... To take place via apoptosis [ 7 ] sirkku Peltonen, Laura,... Directly applicable for human development, physiology, and pathological conditions [ 21 ] for example, the between...
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